Job's syndrome (or hyperimmunoglobulinemia E syndrome) is a rare genetic disease characterized by skin eczema, pyogenic 'cold' abscesses, sinopulmonary recidivous infections and high IgE plasma concentrations. Job's syndrome treatment is not satisfactory and cases studied are still limited. To describe the effects of IVIG therapy in a 37-year-old woman with hyper IgE syndrome and pneumonia. We measured IgE serum by immuno-fluorometric test and neutrophil chemotaxis by migration in a Boyden chamber before and after MG therapy. A moderate dose of IVIG resolved the clinical-radiological signs of the S. aureus bronchopneumonia and improved cytologic and biohumoral parameters. Intravenous immunoglobulins represent a useful treatment for acute pneumonia in Job's syndrome.
MODERATE-DOSE INTRAVENOUS IMMUNOGLOBULIN TREATMENT OF JOB'S SYNDROME
BILORA, FRANCA;POMERRI, FABIO
2000
Abstract
Job's syndrome (or hyperimmunoglobulinemia E syndrome) is a rare genetic disease characterized by skin eczema, pyogenic 'cold' abscesses, sinopulmonary recidivous infections and high IgE plasma concentrations. Job's syndrome treatment is not satisfactory and cases studied are still limited. To describe the effects of IVIG therapy in a 37-year-old woman with hyper IgE syndrome and pneumonia. We measured IgE serum by immuno-fluorometric test and neutrophil chemotaxis by migration in a Boyden chamber before and after MG therapy. A moderate dose of IVIG resolved the clinical-radiological signs of the S. aureus bronchopneumonia and improved cytologic and biohumoral parameters. Intravenous immunoglobulins represent a useful treatment for acute pneumonia in Job's syndrome.File | Dimensione | Formato | |
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